Kazato Yuko, Fujii Katsunori, Oba Hiroshi, Hino Moeko, Ochiai Hidemasa, Uchikawa Hideki, Kohno Yoichi
Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
Brain Dev. 2012 Sep;34(8):696-9. doi: 10.1016/j.braindev.2011.11.001. Epub 2012 Mar 3.
We described a 7-year-old girl with reversible cerebral vasoconstriction syndrome associated with brain parenchymal hemorrhage. She initially presented with high fever and pancytopenia, leading to a diagnosis of most severe type aplastic anemia. We treated her with cyclosporine, methylprednisolone and anti-thymocyte globulin. Thereafter she recurrently complained of a very severe headache called as thunderclap, and finally exhibited loss of consciousness. Brain imaging revealed massive parenchymal hemorrhage between the left occipital and parietal lobes on computed tomography, and diffuse cerebral vasoconstriction on magnetic resonance angiography. The cerebral vasoconstriction resolved within two months, and thus we diagnosed her as having reversible cerebral vasoconstriction syndrome associated with brain parenchymal hemorrhage. This syndrome has been frequently reported in adult females, but rarely in children. However, even in children, a so called thunderclap headache may become a clue for the diagnosis of reversible cerebral vasoconstriction syndrome, especially in cases taking immunosuppressive agents. Immediate magnetic resonance angiography is essential to diagnose this syndrome, and a prompt application of calcium channel inhibitors should be considered to resolve constriction of the vessels and to prevent subsequent brain damage.
我们描述了一名7岁女孩，患有与脑实质出血相关的可逆性脑血管收缩综合征。她最初表现为高热和全血细胞减少，最终被诊断为最严重类型的再生障碍性贫血。我们用环孢素、甲泼尼龙和抗胸腺细胞球蛋白对她进行治疗。此后，她反复诉说有一种极为严重的头痛，称为霹雳样头痛，最终出现意识丧失。脑部影像学检查显示，计算机断层扫描显示左枕叶和顶叶之间有大量实质出血，磁共振血管造影显示弥漫性脑血管收缩。脑血管收缩在两个月内消退，因此我们诊断她患有与脑实质出血相关的可逆性脑血管收缩综合征。这种综合征在成年女性中经常有报道，但在儿童中很少见。然而，即使在儿童中，所谓的霹雳样头痛也可能成为诊断可逆性脑血管收缩综合征的线索，特别是在服用免疫抑制剂的情况下。立即进行磁共振血管造影对于诊断该综合征至关重要，应考虑及时应用钙通道抑制剂以解除血管收缩并预防随后的脑损伤。
